Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related sclerosing cholangitis (SC), an IgG4-related disease with clearly proven autoimmune origin. Differential diagnosis is made even more challenging becau...
Saved in:
Published in | Journal of gastrointestinal and liver diseases : JGLD Vol. 30; no. 3; pp. 398 - 403 |
---|---|
Main Authors | , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Romania
21.09.2021
|
Subjects | |
Online Access | Get full text |
Cover
Loading…
Abstract | Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related sclerosing cholangitis (SC), an IgG4-related disease with clearly proven autoimmune origin. Differential diagnosis is made even more challenging because PSC with increased IgG4 levels (PSC-increased IgG4) also occurs. In order to facilitate their differential diagnosis, we reviewed recent literature regarding the etiologies, identifying characteristics, the most useful diagnostics, treatment, and the progression of these partially similar diseases. It is clear that PSC's pathogenesis differs from that of IgG4-related SC. In any differential diagnosis between PSC and PSC-increased IgG4, high IgG1 and low or normal IgG2 levels are characteristic for patients with PSC. Histological examination of the biliary tree wall in patients with IgG4-related SC typically reveals such changes as storiform fibrosis, obliterative phlebitis, and venulitis. These are absent in PSC-increased IgG4, which is characterized by a typical circular thickness in different parts of the biliary ducts. Finally, PSC is associated with inflammatory bowel disease, which is rare in IgG4-related SC, and more frequently is associated with cholangiocarcinomas and colon cancers. As distinct from IgG4-related SC, PSC is not a primary autoimmune disease. |
---|---|
AbstractList | Background and Aims: Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related sclerosing cholangitis (SC), an IgG4-related disease with clearly proven autoimmune origin. Differential diagnosis is made even more challenging because PSC with increased IgG4 levels (PSC-increased IgG4) also occurs. In order to facilitate their differential diagnosis, we reviewed recent literature regarding the etiologies, identifying characteristics, the most useful diagnostics, treatment, and the progression of these partially similar diseases. It is clear that PSC’s pathogenesis differs from that of IgG4-related SC. In any differential diagnosis between PSC and PSC-increased IgG4, high IgG1 and low or normal IgG2 levels are characteristic for patients with PSC. Histological examination of the biliary tree wall in patients with IgG4-related SC typically reveals such changes as storiform fibrosis, obliterative phlebitis, and venulitis. These are absent in PSC-increased IgG4, which is characterized by a typical circular thickness in different parts of the biliary ducts. Finally, PSC is associated with inflammatory bowel disease, which is rare in IgG4-related SC, and more frequently is associated with cholangiocarcinomas and colon cancers. As distinct from IgG4-related SC, PSC is not a primary autoimmune disease. Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related sclerosing cholangitis (SC), an IgG4-related disease with clearly proven autoimmune origin. Differential diagnosis is made even more challenging because PSC with increased IgG4 levels (PSC-increased IgG4) also occurs. In order to facilitate their differential diagnosis, we reviewed recent literature regarding the etiologies, identifying characteristics, the most useful diagnostics, treatment, and the progression of these partially similar diseases. It is clear that PSC's pathogenesis differs from that of IgG4-related SC. In any differential diagnosis between PSC and PSC-increased IgG4, high IgG1 and low or normal IgG2 levels are characteristic for patients with PSC. Histological examination of the biliary tree wall in patients with IgG4-related SC typically reveals such changes as storiform fibrosis, obliterative phlebitis, and venulitis. These are absent in PSC-increased IgG4, which is characterized by a typical circular thickness in different parts of the biliary ducts. Finally, PSC is associated with inflammatory bowel disease, which is rare in IgG4-related SC, and more frequently is associated with cholangiocarcinomas and colon cancers. As distinct from IgG4-related SC, PSC is not a primary autoimmune disease. |
Author | Uvirova, Magdalena Kojecky, Vladimir Dite, Petr Martinek, Arnost Jabandziev, Petr Dolina, Jiri Hornakova, Lubomira Kunovsky, Lumir |
Author_xml | – sequence: 1 givenname: Lumir surname: Kunovsky fullname: Kunovsky, Lumir email: lumir.kunovsky@gmail.com organization: Department of Gastroenterology and Internal Medicine, Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic. . lumir.kunovsky@gmail.com – sequence: 2 givenname: Petr surname: Dite fullname: Dite, Petr email: pdite.epc@gmail.com organization: Department of Gastroenterology and Internal Medicine, University Hospital Brno, Brno; Department of Internal Medicine, Department of Gastroenterology, University Hospital Ostrava, Ostrava, Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic. . pdite.epc@gmail.com – sequence: 3 givenname: Lubomira surname: Hornakova fullname: Hornakova, Lubomira organization: Department of Gastroenterology and Internal Medicine, University Hospital Brno, Brno; Faculty of Medicine, Masaryk University, Brno, Czech Republic – sequence: 4 givenname: Jiri surname: Dolina fullname: Dolina, Jiri organization: Department of Gastroenterology and Internal Medicine, University Hospital Brno, Brno; Faculty of Medicine, Masaryk University, Brno, Czech Republic – sequence: 5 givenname: Magdalena surname: Uvirova fullname: Uvirova, Magdalena organization: CGB Laboratory a.s., Ostrava, Czech Republic – sequence: 6 givenname: Vladimir surname: Kojecky fullname: Kojecky, Vladimir organization: Faculty of Medicine, Masaryk University, Brno, Department of Internal Medicine, Hospital Zlin, Zlin, Czech Republic – sequence: 7 givenname: Arnost surname: Martinek fullname: Martinek, Arnost organization: Department of Internal Medicine, Department of Gastroenterology, University Hospital Ostrava, Ostrava; Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic – sequence: 8 givenname: Petr surname: Jabandziev fullname: Jabandziev, Petr organization: Faculty of Medicine, Masaryk University, Brno; Department of Pediatrics, University Hospital Brno, Brno; Central European Institute of Technology, Masaryk University, Brno, Czech Republic |
BackLink | https://www.ncbi.nlm.nih.gov/pubmed/34551027$$D View this record in MEDLINE/PubMed |
BookMark | eNo9kMtOwzAQRS1URB-w4QOQ10gBP-NkWbXlIVUqUmHBKnKccXCVOJWdLvh7mhZYzWh0dDX3TNHIdx4QuqXkgUpB-OOubqqEZyK_QBOaCZZQyujotNMkU0yM0TTGHSFpSpS6QmMupKSEqQn6XDprIYDvne6dr_FbcK0O33hrGghdHE6Lr67Rvna9i9iGrsVb17pGB7x0EXSEiDuL54e-c2178IA3wdXOX6NLq5sIN79zhj6eVu-Ll2S9eX5dzNeJYSLtE1lKkSomLZGWEyO4NFBqa3IFkKk0p7kkXFZWUXMspTWnVFeCCKbLrGIl8Bm6P-ea47sxgC325woFJcXJTzH4KQY_R_juDO8PZQvVP_onhP8AkC5j9A |
ContentType | Journal Article |
DBID | CGR CUY CVF ECM EIF NPM AAYXX CITATION |
DOI | 10.15403/jgld-3849 |
DatabaseName | Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed CrossRef |
DatabaseTitle | MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) CrossRef |
DatabaseTitleList | CrossRef MEDLINE |
Database_xml | – sequence: 1 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
DeliveryMethod | fulltext_linktorsrc |
Discipline | Medicine |
EISSN | 1842-1121 |
EndPage | 403 |
ExternalDocumentID | 10_15403_jgld_3849 34551027 |
Genre | Journal Article Review |
GroupedDBID | CGR CUY CVF ECM EIF NPM --- 53G 5GY AAYXX ABDBF ADBBV AENEX ALMA_UNASSIGNED_HOLDINGS BAWUL CITATION DIK EBD EMOBN EOJEC ESX F5P FRP GX1 OBODZ OK1 SV3 TR2 TUS W2D |
ID | FETCH-LOGICAL-c246t-5b546725f05f30c435cebafc97ee8769195035df71c121aa311ad4042ab8d2be3 |
ISSN | 1841-8724 |
IngestDate | Fri Aug 23 03:14:11 EDT 2024 Tue Jul 04 17:29:03 EDT 2023 |
IsDoiOpenAccess | false |
IsOpenAccess | true |
IsPeerReviewed | false |
IsScholarly | true |
Issue | 3 |
Language | English |
LinkModel | OpenURL |
MergedId | FETCHMERGED-LOGICAL-c246t-5b546725f05f30c435cebafc97ee8769195035df71c121aa311ad4042ab8d2be3 |
PMID | 34551027 |
PageCount | 6 |
ParticipantIDs | crossref_primary_10_15403_jgld_3849 pubmed_primary_34551027 |
PublicationCentury | 2000 |
PublicationDate | 2021-09-21 |
PublicationDateYYYYMMDD | 2021-09-21 |
PublicationDate_xml | – month: 09 year: 2021 text: 2021-09-21 day: 21 |
PublicationDecade | 2020 |
PublicationPlace | Romania |
PublicationPlace_xml | – name: Romania |
PublicationTitle | Journal of gastrointestinal and liver diseases : JGLD |
PublicationTitleAlternate | J Gastrointestin Liver Dis |
PublicationYear | 2021 |
SSID | ssj0066077 |
Score | 2.2881281 |
SecondaryResourceType | review_article |
Snippet | Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig) G4-related... Background and Aims: Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease. Differential diagnostics can confuse it with immunoglobulin (Ig)... |
SourceID | crossref pubmed |
SourceType | Aggregation Database Index Database |
StartPage | 398 |
SubjectTerms | Autoimmune Diseases - diagnosis Bile Ducts, Intrahepatic Cholangitis, Sclerosing - diagnosis Diagnosis, Differential Humans Immunoglobulin G |
Title | Differentiating Primary Sclerosing Cholangitis from Similar Diseases of Autoimmune Origin |
URI | https://www.ncbi.nlm.nih.gov/pubmed/34551027 |
Volume | 30 |
hasFullText | 1 |
inHoldings | 1 |
isFullTextHit | |
isPrint | |
link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1ZbxMxELZCkRAviJtCqSzBG9qyh_d6rEgPlbZUaiuFp8jXVgvtLkp2-8B_4b8yYzvOpgWp8GJFKzvRer7MYc_MR8j7HP5mvGQqkFHIA1bxLOCiUoGIZKhL8FCVMtkWx9n-OTuYpJPR6Ncga6nvxJb8-ce6kv-RKjwDuWKV7D9I1n8pPIDPIF8YQcIw3knGY8du0uH-Qsh_4lpHnMI8sH7mSh9jV-Qkque2lOS0vqox83Rsb2ZMJsd237U1ForoD18MUdZfXNYLPu9mLXaYAMXQuC4Dl5jZsbjomZsjhoO9Q59I_Llv2mt3QnvYX9U-GXjsmPmQ08uDC_mSv7fX3M4WLcz3dmNs-IUM7OpZPTyuiCPMrbA10E7DFiwCFWwLp7f04lkcgOMXDdWyu66ph1G70bGJpa125pqZFgm3LQF4otiS4tvFpQqSwrZFXW23fcMM-uREDItw9RTXTnHtPXI_zssUY_u9ic8gyrLQEHv6N3Ldb3HtR_-7K_7OSuRiPJizx-SRkyPdtjh6Qka6eUoeHLnkimfk6w04UQcnuoQTHcCJIpyogxNdwIm2FV3CiVo4PSfnuztnn_YDx7wRyJhlXZCKFAxonFZhWiWhBJdaasErWeZag_kskTs4SVWVRxKkxnkSRVwx0P9cFCoWOnlB1pq20a8IlYWoYiFVXoAjmJUC4iMty4SxFNwnFYl18m6xP9Mf9rWmtyWwTl7arfNzEga-fhjnr--0_g15uATjBlnrZr1-C95kJzaNTGE8Pjn6De3ge5A |
link.rule.ids | 315,786,790,27957,27958 |
linkProvider | Geneva Foundation for Medical Education and Research |
openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Differentiating+Primary+Sclerosing+Cholangitis+from+Similar+Diseases+of+Autoimmune+Origin&rft.jtitle=Journal+of+gastrointestinal+and+liver+diseases+%3A+JGLD&rft.au=Kunovsky%2C+Lumir&rft.au=Dite%2C+Petr&rft.au=Hornakova%2C+Lubomira&rft.au=Dolina%2C+Jiri&rft.date=2021-09-21&rft.issn=1841-8724&rft.eissn=1842-1121&rft.volume=30&rft.issue=3&rft.spage=398&rft.epage=403&rft_id=info:doi/10.15403%2Fjgld-3849&rft.externalDBID=n%2Fa&rft.externalDocID=10_15403_jgld_3849 |
thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=1841-8724&client=summon |
thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=1841-8724&client=summon |
thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=1841-8724&client=summon |